Disorders of sex development(DSD) include anomalies of sex chromosomes,gonads,reproductive ducts,and genitalia.Here,the term "intersex" is avoided because of its imprecision.The purpose of this review is to assist health care professionals in the provision of treatment,education,and support to children born with DSD and to their families.The goal of DSD treatment is to achieve the long-term physical,psychological,and sexual well-being of the patients.In the case of DSD it involves several principles.Providing medical and surgical care to deal with a complication that threatens to the patient's physical well-being;minimizing the potential for the patient and family to feel ashamed,stigmatized,or overly obsessed with genital appearance;delaying elective surgical and hormonal treatments until the patient can actively participate in decision-making;telling the truth to the family and the child and addressing psychosocial distress of the children and their parents.This approach is termed as "patient-centered".

Humans ; Integrative Medicine ; Medicine, Chinese Traditional ; trends

BACKGROUND: Matrix metalloproteinase (MMP) is a kind of protease family, its activity can be inhibited by tissue inhibitor of metalloproteinase (TIMP), especially by TIMP-3.OBJECTIVE: To fully isolate and purify natural TIMP-3, and to create enzyme-linked immunoassay of TIMP-3.DESIGN: Single-sample observation SETTING: Central Laboratory of Shenyang Medical College MATERIALS: Human placenta from Department of Obstetrics & Gynecology, Fengtian Hospital Affiliated to Shenyang Medical College (Informed consent was obtained from the relatives of patients) and MMP-1 from Research Institute. Fuji Chemical Industries, Ltd.METHODS: This experiment was conducted in the Central Laboratory of Shenyang Medical College between March 2001 and May 2002. Firstly,4 mol/L urea Tris-buffer solution (pH8.0) was used to prepare homogenate solution of placenta. Secondly, homogenate solution was performed chromatography through CM52 positive ion-exchange resin and Sephacryl S200 gel filtration. Thirdly, relative molecular weight and purity were detected by SDS- polyacrylamidedel gel electrophoresis (SDS-PAGE).Fourthly, Western blotting was used to identify the characters of purified protein. Fifthly, the inhibitory rate of TIMP-3 to MMP-1 was measured with immumofluorescence method.MAIN OUTCOME MEASURES: ① The relative molecular mass of protein showed by PAGE. ② Results of Western blot. ③ The inhibitory rate of TIMP-3 for MMP-1. ④ The recovery rate of TIMP-3 following purification.RESULTS: ①The isolated and purified TIMP-3 from human placenta consisted of non-glycosylated and glycosylated protein, with relative molecular mass of 24 000 and 27 000, respectively. ② The inhibitory concentration of non-glycosylated and glycosylated TIMP-3 was 1.1×1010 mol/L and 1.2×1010 mol/L respectively to MMP-1. The inhibitory concentration of placenta-derived TIMP-3 was significantly higher than that of recombinant TIMP-3 for MMP-1. ③ The recovery rate of TIMP-3 was 23.4% following two-step chromatography.CONCLUSION: Extracellular matrix of human placenta-derived TIMP-3 consists of non- glycosylated protein and glycosylated protein; Two kinds of purified TIMPs-3 have remarkable inhibitory concentration for MMP-1, and significantly higher in comparison with recombinant metalloproteinase inhibitory factor-3.

Objective To investigate the effects of the silence of teratocarcinoma-derived growth factor-1 ( TDGF-1 ) gene on invasion of human pancreatic cancer cell. Methods Three small interfering RNA (siRNA) targeting for TDGF-1 genes (S1, S2, S3 ) were designed and established, then the gene with the best silencing effects was screened. Human pancreatic cancer cell line PANC1 were transfected by siRNA with different concentrations (3. 125, 6.25, 12.5 nmoL/L), the cells without transfection, and simply treated with liposomes were controls. The expressions of mRNA and protein of TDGF-1 were determined by real time PCR and Western blot assay, respectively. The anchorage-independent growth was examined by clon formation in soft agar, and invasion ability was evaluated by boyden chamber model. PANC1 cells with transfection for 48h were injected into the nude mice to evaluate the invasion ability in vivo. Results The expressions of TDGF-1 mRNA and protein of cells transfected by siRNA were decreased in a dose-and time-dependent manner, which were significantly lower than those in liposomes group. Number of colony formation and transmembrane cell were 19.8 ± 2.2 and 49.8 + 2.6 in the control group, and 5.6 + 1.2 and 8. 1 + 1.1 in the 12.5 nmol/L transfection group. The volumes of tumor 4 weeks after transplation in the control group, liposomes group and the 12.5 nmol/L transfection group were (2.228 ± 0.016 ) cm3, ( 2.186 ± 0.028 )cm3 and ( 0.728 ± 0.023 )cm3. Conclusions TDGF-1 gene silence could inhibit invasion ability of human pancreatic cancer cell PANC1.

Objective The prosthesis used for aortic valve replacement (AVR) can be too small in relation to body size,thus causing valve prosthesis-patient mismatch (PPM).The aim of this article was to summarize the preventive strategy of PPM during AVR.Methods A total of 357 patients [203 males,154 females; mean age (54.9 ± 18.7 ) years ] underwent AVR between February.2010 and December 2011.The weight and body surface area (BSA) of the group is( 60.1 ± 11.4 )kg and (1.67 ± 0.21 )m2 respectively.The aortic valve prosthesis effective orifice area (EOA) was divided by body surface area (BSA) to obtain the EOA index (EOAI).PPM was then defined as none or mild if EOAI was ＞ 0.85 cm2/m2,as moderate for (0.65 - 0.85 ) cm2/m2 and as severe for ＜ 0.65 cm2/m2.To avoid PPM,a simple three-step algorithm was applied:Step 1,Calculate the patient's BSA from weight and height;Step 2,Calculate the minimal valve EOA required based on the BSA to ensure an EOAI ＞0.85 cm2/m2 ; Step 3,Select the type and size of prosthesis that has reference values for EOA greater or equal to the minimal EOA value obtained in step 2.For patients with a small aortic root,the following three methods was used:( 1 ) Replace aortic valve with simple interrupted suture technique ; (2) Apply new type and high-performance prosthetic valves such as St.Jude Medical Regent mechanical valve ; (3) Enlarge the narrowed aortic root when necessary.Results Of all 357 patients,272 patients received mechanical AVR and 85 bioprosthetic AVR.Among the 49 patients who received AVR with simple interrupted suture technique.St.Jude Medical Regent mechanical valve was implanted in 38 patients and the aortic root enlargement was performed in 11 patients.The total prevalence of PPM was 6.4％ and there was no severe PPM.The prevalence of PPM with mechanical AVR and bioprosthetic AVR was 1.8％ and 21.2％ respectively.There were 4 deaths during early period of operation,and the operative mortality was 1.1％.Conclusion Prosthesis-patient mismatch can be effectively prevented at the time of AVR with appropriate measurement.

Objective To summary the experience of management for orthotopic homologous heart transplantation,heart-lung co-transplantation and to discuss the postoperative treatment of short-term complications.Methods Orthotopic homologous heart transplantation and heart-lung co- transplantation on 9 cases were performed from May 2004 to February 2006.The patients were traced closely and all complications were treated in time.Results During hospital stay,hypertension oc- curred in one case,right heart insufficiency in one case,infection of mycetes and cytomegalovirus in 2 cases after the heart transplantation,and in the patient undergoing heart-lung co-transplantation,renal inadequacy,bronchial anastomosis stenosis and fungus infection occurred.All patients obtained symp- tomatic relief before discharge.Conclusion Proper perioperative management,immunosuppressive therapy and management of postoperative complications could reduce mortality and obtain satisfactory therapeutic effect in the near future.

Objective To investigate the clinical manifestations and MR imaging features of olivopontocerebellar atrophy(OPCA).Methods The clinical data and MR findings of 8 patients with OPCA (OPCA group) were retrospectively analyzed,and 75 cases of healthy physical examination people were chosen as control group (they were divided into 21-30 years group,31-40 years group,41-50 years group,51-60 years group,61-70 years group,and 15 cases in each group),all subjects under went pons measurement.Results All 8 patients with OPCA were sporadic cases.The cerebellar symptom,autonomic symptoms and extrapyramidal symptoms were common manifestations.The head MR imaging of OPCA presented with cerebellum and brain stem atrophy,cross sign was the characteristic appearance.Vertical diameter and anteroposterior diameter of the pons in OPCA group patients was (20.63 ± 2.16) and (16.74 ±4.37) mm,compared with control group [(27.14 ±1.73) and (22.28 ± 1.49) mm],there was significant difference (P ＜ 0.01).In control group,vertical diameter and anteroposterior diameter of the pons maximum in 21-30 years group and 31-40 years group,increased with age,the value had been reduced,but there was no significant difference (P ＞ 0.05).Conclusion MR is the first choice for the diagnosis of OPCA,combine with clinical manifestations may improve the diagnostic accuracy.

OBJECTIVE To investigate the trends of the drug-resistance of Staphylococcus in our area and to provide reference basis for rational use of antibiotic.METHODS Referring to Nationwide Rules of Operation in Clinical Laboratory,identification of bacteria was operated.K-B method was used to test drug sensitivity.And the results of drug sensitivity were analyzed.RESULTS None vancomycin-resistant strains were detected in 482 strains of Staphylococcus;the sensitivity rates to amikacin and gentamicin were more than 50.0% and were relatively stable.The sensitivity rate to penicillin was 0 in 2002 and 16.2% in 2006,which showed a tendency towards going up.The sensitivity rate to rifampicin was 56.5% in 2002 and 16.2% in 2006,which showed a decreasing tendency.The sensitivity rates of other drugs were less than 50.0% but stable.CONCLUSIONS It′s better to refer to the results of drug resistance test when treating infection caused by Staphylococcus,and the first choice is vancomycin or amikacin if drug resistance results cannot acquired.

To explore the clinical diagnosis methods of SARS virus infection, and analyze the value of ELISA by which the SARS-Ab was detected. The results show ed that the control group was negative, the positive detection rate was respective ly 2.7% and 92% before and after 12 days when the patients got the SARS in the t est group, and the total positive rate was 94.4%. But the detection rate was 3 1% among 29 suspected patients. In a word, the ELISA method was high specified. Th e detection rate was lower before 12 days and higher after 13 to 16 days when th e patients got the SARS.

Objective To explore the effects ofDachengqiDecoction on morphological changes, lung and spleen indexes, and total IgE levels of mice with allergic asthma; To discuss it relevant mechanism of action.MethodsThe method of OVA sensitization and provocation was used to establish allergic asthma mice models. 20 C57BL/6 mice were randomly divided into control group, model group,Dachengqi Decoction group and dexamethasone positive group. Each medication group was given relevant medicine for gavage. Lung and spleen indexes in each group were detected. The total IgE levels in serum of mice were detected by ELISA, and HE staining was used to conduct pathological observation.Results Compared with control group, the lung index and level of IgE in model group increased significantly and pulmonary histological results showed abundant inflammatory cells infiltrated in the bronchus; compared with the model group, the lung index as well as the levels of IgE decreased significantly in the Dachengqi Decoction and dexamethasone positive group (P<0.05), and the lung tissue organization form was improved significantly.ConclusionDachengqi Decoction has inhibiting effects on pulmonary inflammation of mice with asthma.